The molecular pathogenesis of childhood adrenocortical tumors

Adrenocortical tumors in children and adolescents are rare events. However, the high incidence of adrenocortical tumors in children from the Southern region of Brazil is particularly remarkable, since it has been estimated to be approximately 10-15 times greater than the worldwide incidence. To date, there are no histological or molecular markers that can reliably distinguish benign from malignant adrenocortical tumors. The study of rare genetic syndromes associated with adrenocortical tumors has greatly contributed to the elucidation of sporadic adrenocortical tumorigenesis. Recently, considerable advances toward understanding the molecular mechanisms of adrenocortical tumorigenesis in Brazilian children and adolescents with sporadic adrenocortical tumors have been made. Some of the molecular aspects of sporadic adrenocortical tumors arising in children and adolescents are reviewed here.